Anti-MDA5 positive DM is a distinct clinical subtype of DM characterized by autoantibodies targeting MDA5, most often associated with hypo or amyopathic features, severe cutaneous manifestations including skin ulcers and a high risk of rapidly-progressive interstitial lung disease (RP-ILD) development (3, 4). The gene discussed is IFIH1; the disease is retinitis pigmentosa 1.